What it is
Systemic sclerosis (scleroderma) is a rare autoimmune disease in which the immune system, blood vessels, and connective tissue are affected, leading to progressive scarring (fibrosis) of the skin and internal organs.
Signs and symptoms
Pulmonary arterial hypertension
High blood pressure in the lung arteries (pulmonary arterial hypertension) is a serious complication and a major cause of harm in systemic sclerosis.
Raynaud phenomenon
Raynaud's phenomenon, where fingers change colour and go numb in the cold, is an early and near-universal feature, reflecting the blood-vessel involvement of the disease.
Digital ulcer
Painful sores at the fingertips (digital ulcers) can develop because of the reduced blood flow caused by the disease's blood-vessel damage.
Sclerodactyly
Tightening and hardening of the skin of the fingers (sclerodactyly) is a hallmark skin change, often following early puffy swelling of the fingers.
Treatment and management
What the research describes, not a recommendation. Treatment decisions belong with your clinician.
This covers treatments that appear in the published research mapped here. Investigational and experimental therapies are not included, so their absence is a boundary of this map, not a sign they do not exist.
Mycophenolate mofetil
Mycophenolate mofetil is an immune-suppressing medicine used as a backbone treatment for the lung scarring (interstitial lung disease) that can occur in systemic sclerosis.
Used to help with: Systemic sclerosis.
“Current evidence suggests that combined immunosuppressive therapy with mycophenolate mofetil (MMF)-or less frequently cyclophosphamide (CYC)- plus a biologic agent-such as rituximab (RTX) or tocilizumab (TCZ)-is a rational and effective strategy in inflammatory, progressive systemic sclerosis (SSc)-associated interstitial lung disease (ILD), particularly when ILD coexists with other SSc domains encompassing skin involvement, arthritis, and myositis.”
Turn this into questions for your doctor
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How to read the evidence labels
Where this comes from
This guide is built from 2 published source(s). Every claim above links back to one of them. Click any source ID to read the original on PubMed.